Chapelon-Abric Catherine, de Zuttere Dominique, Duhaut Pierre, Veyssier Pierre, Wechsler Bertrand, Huong Du Le Thi, de Gennes Christian, Papo Thomas, Blétry Olivier, Godeau Pierre, Piette Jean-Charles
From Service de Médecine Interne (CC-A, PD, BW, DLTH, CdG, TP, PG, JCP), Hôpital de la Pitié, Paris; Service de Physiologie-Explorations Fonctionnelles (DdZ), Hôpital Bichât, Paris; Service de Médecine Interne (PV), Centre Hospitalier de Compiegne, Compiegne; and Service de Médecine Interne (OB), Hôpital Foch, Suresnes, France.
Medicine (Baltimore). 2004 Nov;83(6):315-334. doi: 10.1097/01.md.0000145367.17934.75.
This retrospective study concerned 18 female and 23 male patients with cardiac sarcoidosis (CS). The average age at CS diagnosis was 38 years. CS was observed in white (73% of cases) and in black or Caribbean patients (27% of cases). All patients had extracardiac histologic proof of sarcoid tissue. In 63% of cases, the CS arose during the follow-up of systemic sarcoidosis. Systemic sarcoidosis was not specific except for a high frequency of neurosarcoidosis. Revealing cardiac signs were clinical in 63% of cases and electrical in 22%. In most patients these signs were associated with an abnormal echocardiography (77%) and/or a defect on thallium-201 or sestamibi imaging (75%). Thirty-nine patients received steroid therapy (initial dose mostly equal to 1 mg/kg per day), associated in 13 cases with another immunosuppressive treatment. In 26% of cases the immunosuppressive treatment was associated with a specific cardiac treatment. In the long-term follow-up (average follow-up, 58 mo), 87% of the cases showed an improvement, and 54% were cured from a clinical and laboratory point of view (electrocardiogram, 24-hour monitoring, echocardiography, radionuclide imaging). There was no sudden death. Two patients worsened, which can be explained in 1 case by very late treatment and in the other case by lack of treatment, except for a pacemaker. Our experience leads us to treat CS with corticosteroids as soon as possible and to use another immunosuppressive treatment where there is an insufficient therapeutic response or where there are contraindications to corticosteroids.
这项回顾性研究涉及18例女性和23例男性心脏结节病（CS）患者。CS诊断时的平均年龄为38岁。在白人患者（占病例的73%）和黑人或加勒比患者（占病例的27%）中均观察到CS。所有患者均有心脏外结节病组织的组织学证据。63%的病例中，CS在系统性结节病的随访期间出现。系统性结节病并无特异性，只是神经结节病的发生率较高。63%的病例中，首发心脏症状为临床症状，22%为电生理症状。大多数患者这些症状与超声心动图异常（77%）和/或铊-201或司他米比显像缺陷（75%）相关。39例患者接受了类固醇治疗（初始剂量大多等于每日1mg/kg），13例联合了另一种免疫抑制治疗。26%的病例中，免疫抑制治疗联合了特异性心脏治疗。在长期随访（平均随访58个月）中，87%的病例有改善，54%从临床和实验室角度（心电图、24小时监测、超声心动图、放射性核素显像）治愈。无猝死发生。2例患者病情恶化，1例可解释为治疗过晚，另1例除植入起搏器外未接受治疗。我们的经验使我们建议尽快用皮质类固醇治疗CS，并在治疗反应不足或存在皮质类固醇禁忌证时使用另一种免疫抑制治疗。
